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Cardiomyopathies 12/98 medslides.com
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Definition “A primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures … myocardial infarction, systemic hypertension, valvular stenosis or regurgitation” 12/98 medslides.com
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WHO Classification Unknown cause (primary)
Dilated Hypertrophic Restrictive unclassified Specific heart muscle disease (secondary) Infective Metabolic Systemic disease Heredofamilial Sensitivity Toxic Br Heart J 1980; 44: 12/98 medslides.com
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Functional Classification
Dilatated (congestive, DCM, IDC) ventricular enlargement and syst dysfunction Hypertrophic (IHSS, HCM, HOCM) inappropriate myocardial hypertrophy in the absence of HTN or aortic stenosis Restrictive (infiltrative) abnormal filling and diastolic function 12/98 medslides.com
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Idiopathic Dilated Cardiomyopathy
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IDC - Definition a disease of unknown etiology that principally affects the myocardium LV dilatation and systolic dysfunction pathology increased heart size and weight ventricular dilatation, normal wall thickness heart dysfunction out of portion to fibrosis 12/98 medslides.com
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Incidence and Prognosis
3-10 cases per 100,000 20,000 new cases per year in the U.S.A. death from progressive pump failure 1-year 25% 2-year % 5-year % stabilization observed in 20-50% of patient complete recovery is rare 12/98 medslides.com
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Idiopathic Dilated Cardiomyopathy Observed Survival of 104 Patients
Years Am J Cardiol 1981; 47:525 12/98 medslides.com
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Clinical Manifestations
Highest incidence in middle age blacks 2x more frequent than whites men 3x more frequent than women symptoms may be gradual in onset acute presentation misdiagnosed as viral URI in young adults uncommon to find specific myocardial disease on endomyocardial biopsy 12/98 medslides.com
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History and Physical Examination
Symptoms of heart failure pulmonary congestion (left HF) dyspnea (rest, exertional, nocturnal), orthpnea systemic congestion (right HF) edema, nausea, abdominal pain, nocturia low cardiac output fatigue and weakness hypotension, tachycardia, tachypnea, JVD 12/98 medslides.com
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Cardiac Imaging Chest radiogram Electrocardiogram
24-hour ambulatory ECG (Holter) lightheadedness, palpitation, syncope Two-dimensional echocardiogram Radionuclide ventriculography Cardiac catheterization age >40, ischemic history, high risk profile, abnormal ECG 12/98 medslides.com
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Management of DCM Limit activity based on functional status
salt restriction of a 2-g Na+ (5g NaCl) diet fluid restriction for significant low Na+ initiate medical therapy ACE inhibitors, diuretics digoxin, carvedilol hydralazine / nitrate combination 12/98 medslides.com
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Management of DCM consider adding ß-blocking agents if symptoms persists anticoagulation for EF <30%, history of thromboemoli, presence of mural thrombi intravenous dopamine, dobutamine and/or phosphodiesterase inhibitors cardiac transplantation 12/98 medslides.com
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Hypertrophic Cardiomyopathy
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Hypertrophic Cardiomyopathy
First described by the French and Germans around 1900 uncommon with occurrence of 0.02 to 0.2% a hypertrophied and non-dilated left ventricle in the absence of another disease small LV cavity, asymmetrical septal hypertrophy (ASH), systolic anterior motion of the mitral valve leaflet (SAM) 12/98 medslides.com
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65% 35% 10% 12/98 medslides.com
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Familial HCM First reported by Seidman et al in 1989
occurs as autosomal dominant in 50% 5 different genes on at least 4 chromosome with over 3 dozen mutations chromosome 14 (myosin) chromosome 1 (troponin T) chromosome 15 (tropomyosin) chromosome 11 (?) 12/98 medslides.com
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Pathophysiology Systole Diastole Myocardial ischemia
dynamic outflow tract gradient Diastole impaired diastolic filling, filling pressure Myocardial ischemia muscle mass, filling pressure, O2 demand vasodilator reserve, capillary density abnormal intramural coronary arteries systolic compression of arteries 12/98 medslides.com
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Clinical Manifestation
Asymptomatic, echocardiographic finding Symptomatic dyspnea in 90% angina pectoris in 75% fatigue, pre-syncope, syncope risk of SCD in children and adolescents palpitation, PND, CHF, dizziness less frequent 12/98 medslides.com
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Increase in Gradient and Murmur
Contractility Preload Afterload valsalva (strain) --- standing --- -- postextrasystole -- isoproterenol digitalis -- amyl nitrite -- nitroglycerine --- exercise tachycardia -- hypovolemia 12/98 medslides.com
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Decrease in Gradient and Murmur
Contractility Preload Afterload Mueller meneuver --- valsalva (overshoot) --- squatting --- passive leg elevation --- --phenylephrine beta-blocker -- general anesthesia isometric grip 12/98 medslides.com
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Natural History annual mortality 3% in referral centers probably closer to 1% for all patients risk of SCD higher in children may be as high as 6% per year majority have progressive hypertrophy clinical deterioration usually is slow progression to DCM occurs in 10-15% 12/98 medslides.com
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Risk Factors for SCD Young age (<30 years)
“Malignant” family history of sudden death Gene mutations prone to SCD (ex. Arg403Gln) Aborted sudden cardiac death Sustained VT or SVT Recurrent syncope in the young Nonsustained VT (Holter Monitoring) Brady arrhythmias (occult conduction disease) 12/98 Br Heart J 1994; 72:S13 medslides.com
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Recommendations for Athletic Activity
Avoid most competitive sports (whether or not symptoms and/or outflow gradient are present) Low-risk older patients (>30 yrs) may participate in athletic activity if all of the following are absent 12/98 medslides.com
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Recommendations for Athletic Activity
Low-risk older patients (>30 yrs) may participate in athletic activity if all of the following are absent ventricular tachycardia on Holter monitoring family history of sudden death due to HCM history of syncope or episode of impaired consciousness severe hemdynamic abnormalities, gradient 50 mmHg exercise induced hypotension moderate or sever mitral regurgitation enlarged left atrium (50 mm) paroxysmal atrial fibrillation abnormal myocardial perfusion 12/98 medslides.com
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Management beta-adrenergic blockers calcium antagonist disopyramide
amiodarone, sotolol DDD pacing myotomy-myectomy plication of the anterior mitral leaflet 12/98 medslides.com
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Other Causes of Hypertrophy
Clinical mimics glycogen storage, infants of diabetic mothers, amyloid Genetic Noonan’s, Friedreich’s ataxia, Familial restrictive cardiomyopathy with disarray Exaggerated physiologic response Afro-Caribbean hypertension, old age hypertrophy, athlete’s heart 12/98 medslides.com
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HCM vs Athlete’s Heart HCM Athlete
+ Unusual pattern of LVH LV cavity <45 mm LV cavity >55 mm LA enlargement Bizarre ECG paterns Abnormal LV filling Female gender thickness with deconditioning + + Family history of HCM - 12/98 Circulation 1995; 91:1596 medslides.com
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Hypertensive HCM of the Elderly
Characteristics modest concentric LV hypertrophy (<22 mm) small LV cavity size associated hypertension ventricular morphology greatly distorted with reduced outflow tract sigmoid septum and “grandma SAM” 12/98 medslides.com
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Restrictive Cardiomyopathy
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Restrictive Cardiomyopathies
Hallmark: abnormal diastolic function rigid ventricular wall with impaired ventricular filling bear some functional resemblance to constrictive pericarditis importance lies in its differentiation from operable constrictive pericarditis 12/98 medslides.com
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Exclusion “Guidelines”
LV end-diastolic dimensions 7 cm Myocardial wall thickness 1.7 cm LV end-diastolic volume 150 mL/m2 LV ejection fraction < 20% 12/98 medslides.com
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Classification Idiopathic Myocardial 1. Noninfiltrative Scleroderma
Amyloid Sarcoid Gaucher disease Hurler disease 3. Storage Disease Hemochromatosis Fabry disease Glycogen storage Endomyocardial endomyocardial fibrosis Hyperesinophilic synd Carcinoid metastatic malignancies radiation, anthracycline 12/98 medslides.com
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Clinical Manifestations
Symptoms of right and left heart failure Jugular Venous Pulse prominent x and y descents Echo-Doppler abnormal mitral inflow pattern prominent E wave (rapid diastolic filling) reduced deceleration time ( LA pressure) 12/98 medslides.com
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Constrictive - Restrictive Pattern “Square-Root Sign” or “Dip-and-Plateau”
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Restriction vs Constriction
History provide can important clues Constrictive pericarditis history of TB, trauma, pericarditis, sollagen vascular disorders Restrictive cardiomyopathy amyloidosis, hemochromatosis Mixed mediastinal radiation, cardiac surgery 12/98 medslides.com
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Treatment No satisfactory medical therapy
Drug therapy must be used with caution diuretics for extremely high filling prssures vasodilators may decrease filling pressure ? Calcium channel blockers to improve diastolic compliance digitalis and other inotropic agents are not indicated 12/98 medslides.com
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